Symptomatic Chiari type 1 malformation associated with acromegaly: A case report.

BACKGROUND: Here, we report a patient who presented with both symptomatic acromegaly and symptomatic Chiari I malformation (CM1) with a C2-T5 syrinx. CASE DESCRIPTION: A 63-year-old female presented with bilateral arm dysesthesias and back pain. For approximately the past 30 years, she had chronic signs of acromegaly (i.e. an enlarged forehead, jaw, and nose, and enlarged hands and feet). When the cervical magnetic resonance showed a CM1 (tonsillar herniation) with C2-T5 syringomyelia, she underwent foramen magnum decompression and C1 posterior arch resection. Postoperatively, she was asymptomatic. The added finding of a growth hormone (GH)-producing pituitary lesion was treated medically with endocrine therapy, as she had incidentally required surgery/chemotherapy for a newly diagnosed colon cancer. CONCLUSION: Symptomatic CM1, syrinx, and acromegaly may occur together. Appropriately treatment may include a suboccipital decompression, and C1 arch resection surgery, followed by either surgical or medical treatment for the GH-producing pituitary adenoma.

Pituitary apoplexy after cardiac surgery in a 14-year-old girl with Carney complex: a case report.

A 14-year-old girl was referred to our department because of headache and visual impairment following the resection of recurrent cardiac myxoma. Head magnetic resonance imaging (MRI) scan detected an intra- and supra-sellar tumor. Moreover, the patient showed the presence of spotty skin pigmentations on her cheeks and lower lip. Blood examination revealed hypothyrotropinemia, and ultrasonography results revealed multiple thyroid nodules. She was diagnosed with Carney complex (CNC). Her pituitary tumor was suspected as growth hormone (GH)-secreting adenoma, because overgrowth was observed in the patient. However, biochemical examinations, including oral glucose tolerance test, failed to show the characteristic findings of GH-secreting adenoma. In contrast, insulin tolerance test showed GH deficiency. Her visual impairment improved without performing decompression surgery, and the tumor size decreased, as per the MRI findings. Based on clinical course, the patient was diagnosed with pituitary apoplexy in pituitary adenoma, following which she was discharged. At 3 months after discharge, thyrotropin-releasing hormone loading test performed revealed low thyrotropin-stimulating hormone and thyroid hormone levels, and the patient was in a depressed mood. Therefore, l-T4 replacement was initiated, following which her GH secretory capacity gradually improved. Here, we report, to the best of our knowledge, the first case of a patient with pituitary apoplexy in CNC. Such condition must be identified in young patients with recurrent cardiac myxoma, and examinations, such as head MRI, must be performed.

Unusual Presentations of Pediatric Skull Hemangiomas: Report of Two Cases.

BACKGROUND: Intraosseous hemangioma is a rare bone tumor, accounting for 0.7%-1.0% of all bone tumors. It can occur at any age, but only 9% of cases are younger than 10 years old. Although this tumor is usually slow-growing and clinically silent, we experienced 2 pediatric patients undergoing surgery for skull hemangioma who presented with uncommon clinical manifestations. CASE DECRIPTION: Case 1 was a 9-year-old boy who presented with sudden onset of headache and was referred to our hospital. Radiologic images revealed an osteolytic oval lesion in the right parietal bone and acute subdural hemorrhage in the right cerebral hemisphere. The right parietal lesion was removed surgically. The lesion was found to have grown into the dura and to be adherent to the pia matter. The removed lesion was histologically confirmed to be a hemangioma. Case 2 was an 8-year-old girl who was referred to our hospital with an elastic mass that had been slowly enlarging for 7 years. Radiologic images revealed an osteolytic oval lesion in the right parietal bone. Surgical removal was thus planned. The lesion was found to be attached to the dura, and we removed the lesion with the surrounding bone and attached dura. Histologic examination confirmed the lesion to be a hemangioma. CONCLUSIONS: Although skull hemangiomas show clinical heterogeneity, surgical removal is usually diagnostic and leads to good patient outcomes. On occasion, however, this tumor causes secondary changes in the dura, such that dural incision and dural plasty should be planned in advance of lesion removal.

A Case of Childhood-Onset Giant Cell Tumor that Caused Optic Nerve Atrophy in Both Eyes.

PURPOSE: The purpose of this study was to report the case of a female patient who had a giant cell tumor in the paranasal sinus during childhood, and while undergoing multiple resection surgeries experienced optic atrophy in both eyes. CASE PRESENTATION: This study involved a 35-year-old woman who was previously diagnosed with a giant cell tumor of the paranasal sinus bone at age 13. A CT scan revealed a large tumor extending from the sphenoid sinus to the ethmoid sinus. At age 14, a tumor resection was performed in conjunction with radiation therapy. However, after resection and radiation therapy there were repeated recurrences, and additional resections were performed. Ophthalmically, there was marked optical atrophy in both eyes, and Goldmann visual field perimetry revealed that only the arcuate peripheral area remained on the nasal side of the right eye, and that there were dark spots in the paracentral area of the left eye. CONCLUSIONS: In this case, a large giant cell tumor occurred in the sphenoid sinus and ethmoid sinus during childhood, and it is thought that optic atrophy was caused by compressive optic neuropathy. The sphenoid sinus and ethmoid sinuses are anatomically close to the optic nerve, and when a tumor grows larger at this site it can easily put pressure on the optic nerve. Therefore, early detection and treatment are important.

Treatment of a skull-base giant cell tumor with endoscopic endonasal resection and denosumab: case report.

A 34-year-old man with a 1-week history of diplopia was referred to the authors' hospital. Neurological examination revealed left abducens nerve palsy. Computed tomography showed a lesion in the left sphenoid sinus involving the medial wall of the left internal carotid artery (ICA) and osteolytic change at the clivus bordering the lesion. Magnetic resonance imaging demonstrated an extensive soft-tissue mass occupying the left sphenoid sinus. Surgical intervention by the endoscopic transnasal method allowed most of the lesion to be removed. Only the portion attached to the medial wall of the ICA was not removed. Postoperatively, the lesion was diagnosed as a giant cell tumor (GCT) and the patient received 120 mg of subcutaneous denosumab every 4 weeks, with additional doses on Days 8 and 15 during the first month of therapy. MRI a week after starting denosumab revealed shrinkage of the initially fast-growing residual tumor. The patient was discharged upon completion of the third denosumab administration. GCT is an aggressive stromal tumor developing mainly in young adults. Complete resection is recommended for GCT in the literature. However, size and location of the CGT often limit this approach. Various adjuvant treatments for skull base GCTs have been reported, including radiation and chemotherapy. However, the roles of adjuvant therapies have yet to be clearly defined. Denosumab, a monoclonal antibody, was recently approved for GCT in several countries. Denosumab may permit less invasive treatments for patients with GCTs while avoiding deleterious outcomes, and may also limit disease progression and recurrence.

Neurological symptoms in a patient with isolated adrenocorticotropin deficiency: case report and literature review.

BACKGROUND: Isolated adrenocorticotropic hormone (ACTH) deficiency is a pituitary disorder characterized by reduction only in the secretion of ACTH. Although the underlying mechanism remains to be elucidated, numbers of cases with this entity have been increasing. We experienced a case presenting with gait disturbance necessitating differential diagnosis from idiopathic normal pressure hydrocephalus (iNPH). CASE PRESENTATION: A 69-year-old female with a complaint of difficulty walking and suspected to have iNPH at a prior hospital was referred to our department. For the prior three years, she had suffered from a progressive gait disturbance. Magnetic resonance imaging (MRI) revealed global ventricular dilatation. The typical features of the gait in iNPH cases were all identifiable. Neuropsychological dementia scale tests showed deterioration. However, the major feature of a disproportionately enlarged subarachnoid-space on MRI was not obvious. The patient developed progressively worsening fatigue during hospitalization. Her symptoms resembled those of hypothalamic-pituitary tumor patients. Serum ACTH and cortisol levels were low. While corticotrophin releasing hormone stress tests showed no response, other stress tests using thyrotropin releasing hormone, luteinizing hormone releasing hormone, and growth hormone releasing hormone yielded normal responses, indicating a diagnosis of isolated ACTH deficiency. We initiated corticosteroid therapy, and her gait disturbance improved promptly. CONCLUSION: Isolated ACTH deficiency may have major significance to the differential diagnosis of iNPH. Early consideration of this entity is anticipated to facilitate making an early diagnosis.

Strategies to prevent positioning-related complications associated with the lateral suboccipital approach.

The lateral positioning used for the lateral suboccipital surgical approach is associated with various pathophysiologic complications. Strategies to avoid complications including an excessive load on the cervical vertebra and countermeasures against pressure ulcer development are needed. We retrospectively investigated positioning-related complications in 71 patients with cerebellopontine angle lesions undergoing surgery in our department between January 2003 and December 2010 using the lateral suboccipital approach. One patient postoperatively developed rhabdomyolysis, and another presented with transient peroneal nerve palsy on the unaffected side. Stage I and II pressure ulcers were noted in 22 and 12 patients, respectively, although neither stage III nor more severe pressure ulcers occurred. No patients experienced cervical vertebra and spinal cord impairments, brachial plexus palsy, or ulnar nerve palsy associated with rotation and flexion of the neck. Strategies to prevent positioning-related complications, associated with lateral positioning for the lateral suboccipital surgical approach, include the following: atraumatic fixation of the neck focusing on jugular venous perfusion and airway pressure, trunk rotation, and sufficient relief of weightbearing and protection of nerves including the peripheral nerves of all four extremities.

[Munchausen syndrome, a factitious injury, presenting brain abscess and intraventricular hemorrhage: a case report].

Munchausen syndrome is a factitious disorder. Patients sometimes inflict injury on themselves in order to assume a sick role. The authors report a patient with Munchausen syndrome suffered from brain abscess, reopened wound and intraventricular hemorrhage. A 64-year-old male was admitted to our hospital after head injury. CT and MR imaging revealed a mass with surrounding edema in the right frontal lobe. The mass was surgically removed, and diagnosed as brain abscess. During the surgery, the authors noticed a small bone defect in the frontal bone above the brain abscess; therefore, we considered that head injury just concerned this lesion. There were no particular clues leading to other possible pathologies. After the first surgery, the patient presented atypical seizures several times. Once we discharged him from our hospital, we hospitalized him again because the wound had reopened. A subsequent operation was needed, and we removed the bone flap which we considered the origin of the infection. After the second surgery, he stabbed a nail into his head where the bone had been removed due to the previous surgery, and presented intraventricular hemorrhage. The hemorrhage decreased in size through non-surgical treatment and he was referred to the psychiatry department under a diagnosis of Munchausen syndrome. Diagnosis of this entity is difficult and often made at the later stage of hospitalization, because patients present a variety of complaints and clinical symptoms, which are hardly proved factitious. Early consideration of this syndrome will offer an early and accurate diagnosis, and is mandatory for a good prognosis.

Usefulness of phase contrast cine mode magnetic resonance imaging for surgical decision making in patients with hydrocephalus combined with achondroplasia. Case report.

A 1-year 9-month-old girl presented with achondroplasia. Serial magnetic resonance (MR) imaging demonstrated mild compression of the medulla oblongata by the occipital bone, macrocrania, and progressive hydrocephalus. Cerebrospinal fluid (CSF) flow study using MR imaging clearly demonstrated CSF flow disturbance at the cervicospinal junction. Foramen magnum decompression was performed for her hydrocephalus and compressed medulla. Postoperative CSF flow study demonstrated improvement of CSF flow at the craniocervical junction. The patient has remained in a stable condition for 7 months postoperatively. Achondroplasia represents hydrocephalus or medullary compression caused by narrowed foramen magnum, which can result in sudden death in some infants. Surgical indications and methods for hydrocephalus combined with achondroplasia remain controversial because the natural history of the hydrocephalus has remained unclear. CSF flow study using MR imaging can provide useful information regarding the surgical indication and methods for the treatment of hydrocephalus combined with achondroplasia.

Surgical strategy of brain tumors adjacent to the optic radiation using diffusion tensor imaging-based tractography.

The present study evaluated the efficacy of diffusion tensor imaging (DTI)-based tractography in the surgery of brain tumors adjacent to the optic radiation. Of the 14 surgical cases included, 11 had metastatic brain tumors and 3 cerebral gliomas. Additionally, 4 of the 14 patients had pre-operative visual field defects, while the remaining 10 patients experienced no visual impairment. The optic radiations on the lesion side were evident in all 14 patients. On the basis of these tractographic findings, we employed optimal surgical approaches in each patient to avoid injury to the eloquent neural structures, including optic radiation, during surgery. Successful surgical resection was performed in all 14 patients. Of the 14 patients, 2 with visual field defects during the pre-operative period showed improvement in their visual field, and the remaining 12 patients experienced no visual deterioration. DTI-based tractography thus is a feasible modality for the surgical planning of brain lesions adjacent to the optic radiation.

Usefulness and limitations of fluorine-18-fluorodeoxyglucose positron emission tomography for the detection of malignancy of orbital tumors.

Fluorine-18-fluorodeoxyglucose positron emission tomography ([18F]FDG PET) was assessed as a method for providing information about the malignancy of orbital tumors. Twelve patients with 13 orbital tumors underwent [18F]FDG PET followed by biopsy or tumor removal via a transcranial approach. The accumulation ratio between the tumor and the contralateral normal tissue (T/N ratio) was calculated for 10 of the 13 lesions. The T/N ratio in benign lesions was compared with that in malignant tumors. Histological examination identified 7 lesions as malignant: anaplastic astrocytoma of the optic nerve in 1 patient, which recurred as glioblastoma of the optic nerve, malignant lymphoma of mucosa-associated lymphoid tissue type in 1 patient, malignant melanoma in 1 patient, adenoid cystic carcinoma in 2 patients, and adenocarcinoma (unknown origin) in 1 patient. The T/N ratio was 1.06 +/- 0.03 (mean +/- standard deviation) in benign tumors, and significantly higher at 1.81 +/- 0.27 in malignant tumors (p = 0.0027). Both patterns of high and iso uptake of [18F]FDG were found in orbital pseudotumor. [18F]FDG PET can determine the malignancy of orbital tumors, but cannot distinguish malignant tumor from inflammatory disease such as pseudotumor.

Metastatic skull tumor from cholangiocarcinoma. Case report.

A 67-year-old female presented with a rare metastatic skull tumor from cholangiocarcinoma (CCC) manifesting as a progressive painful subcutaneous tumor. Computed tomography and magnetic resonance (MR) imaging revealed an osteolytic tumor attached to the sigmoid sinus and mastoid sinus. Mass reduction of the tumor was performed and radiotherapy applied to the postoperative cavity. The symptoms resolved following the surgical intervention. The skull metastasis from CCC appeared as heterogeneous intensity on MR imaging reflecting the honeycomb structure. Surgery should be considered to relieve symptoms and improve the patient's quality of life, if there is a low risk of morbidity or mortality. The present case indicates another metastatic pathway through the vertebral plexus in the clinical course of CCC.

[Orbital schwannoma developing from the superior branch of the oculomotor nerve--case report].

Orbital schwannoma accounts for 1-6% of all orbital tumors and it is not easy to identify the origin of the tumor because of the anatomy of the orbit. A case of orbital schwannoma developing from the superior branch of the oculomotor nerve is reported. A 74-year-old female was admitted to our hospital with the chief complaint of left exophthalmus. MR imaging revealed a space-occupying lesion in the left orbit. Operation was performed through the transcranial approach. It was noticed that the tumor was developing from the nerve that runs into the superior rectus muscle or the levator palpebrae superioris muscle, and it was diagnosed as a schwannoma on frozen section histology during surgery. The tumor was successfully removed and, postoperatively, no additional defects occurred. Careful consideration of microsurgical anatomy is essential for surgery of orbital schwannoma especially when it's necessary to cut the nerves.

Positron emission tomography elucidates transport system and tumor proliferation in meningiomas.

To test the in vivo transport system and tumor proliferation of meningiomas, in comparison with gliomas, 25 patients with meningiomas and 8 gliomas underwent quantitative kinetic analysis of ([18F])fluoro-2-deoxy-D-glucose (FDG) - positron emission tomography (PET) imaging and immunohistochemical study. Kinetic analysis was obtained by calculation of the rate constants: K1 (ml/g/min), which represents the transport of FDG from plasma to tissue; k2 (min(-1)), which demonstrates the transport back from tissue to plasma; and k3 (min(-1)), an indicator of glucose metabolism, using Gjedde's plot methods in a three-compartment model. Surgical specimens were evaluated by means of three different methods: i) immunoreactivity to vascular endothelial growth factor (VEGF) and glucose transporter-1 (Glut-1), representing the permeability of tumor vessels; ii) immunostaining for von Willebrand Factor (vWF), reflecting vascular surface areas of arterioles; and iii) the MIB-1 labeling index (MIB-1 LI), representing the proliferative potential. K1 was higher in meningiomas than in gliomas and was higher in atypical than in benign meningiomas. k3 was correlated with MIB-1 LI in meningiomas, but not in gliomas. Immunohistochemically, meningiomas were less reactive to VEGF or Glut-1 than gliomas but atypical meningiomas stained more intensely than benign meningiomas. The vascular surface area was significantly larger in meningiomas than in gliomas and atypical meningiomas had high values for both permeability and surface area than benign meningiomas. High values for K1 and k3 indicate the aggressive proliferation of meningiomas and, in atypical meningiomas, the synergistic interaction of the high permeability and the large surface area yielded conditions conducive to glucose metabolism and tumor proliferation. Evaluation of K1 and k3 facilitates to predict the tumor aggressiveness and to optimize the surgical management.